Supranuclear Facial Palsy Research Articles

Moyamoya Disease From an Otolaryngologist’s Perspective: A Rare Case

Moyamoya disease is characterized by narrowing of cerebral blood vessels and is progressive in nature. We present the case of a 21-year-old female patient who was a known case of Down’s syndrome and presented with right-sided facial muscle weakness and was initially suspected of having cholesteatoma, but no evidence for the same was found after extensive radiological investigations. The suspicion of a central nervous system pathology was raised due to the clinical findings of dysphasia and supranuclear facial palsy. Magnetic-resonance angiography (MRA) was suggestive of findings of early Moyamoya disease. After a Neurologist consultation, the patient was started on conservative management with anti-platelet drugs and Statins and had symptomatic improvement. The patient was advised regular follow-up and had no fresh episodes of similar complaints.

A rare case of concomitant sicca keratopathy and ipsilateral central facial palsy in Wallenberg's dorsolateral medullary syndrome.

Objective: To describe a patient with a right-sided supranuclear facial palsy and concomitant sicca keratopathy of the right eye following right-sided dorsolateral medullary infarction. Methods: Our patient underwent a complete ophthalmologic and neurologic examination including biomicroscopy, fundus examination, cranial nerve examination, Shirmer I test, and magnetic resonance imaging of the brain.Results: A 61-year-old woman presented in emergency with a central facial nerve palsy on the right side and truncal ataxia. Neurologic assessment revealed a concurrent dysphagia, dysarthria, hypoesthesia of the right face, and weakness of the right upper limb. Magnetic resonance imaging of the brain showed an old left-sided cerebellar infarction, but a recent ischemic infarction at the level of the right dorsolateral medulla oblongata was the cause of our patient’s current problems. One month after diagnosis of the right-sided dorsolateral medullary syndrome, there were complaints of ocular irritation and a diminished visual acuity in the right eye. Biomicroscopy showed a sicca keratopathy with nearly complete absence of tear secretion on the Shirmer I test, but with normal eye closure and preserved corneal reflexes and sensitivity.Conclusion: A dorsolateral medullary syndrome can have a variable expression in symptomatology. Our case is special because of the combination of an ipsilateral supranuclear facial palsy with normal upper facial muscle function together with an ipsilateral sicca keratopathy as a result of a nearly absent tear secretion. We hypothesized that the mechanism underlying the patient’s sicca keratopathy ipsilateral to the supranuclear facial palsy involved the superior salivatory nucleus, which is situated in the caudal pons inferiorly of the motor facial nucleus and is most probably affected by a superior extension of the infarcted area in the right medulla oblongata.

White matter lesions in a young HIV–positive immunocompetent patient without cerebrovascular risc factors due to an ICA–thrombus

Sirs: White matter lesions in HIVpositive patients may have various causes. Besides inflammatory and neoplastic diseases [1], vascular processes should be considered. Hemostasis disturbances and thrombotic microangiopathy have been described in HIV-infected patients [5, 6, 8] and venous or arterial thrombosis have been observed in HIV-infected patients [3, 10]. To the best of our knowledge, internal carotid artery (ICA) thrombosis has not yet been described in HIVpositive patients in the absence of other major thrombophilic factors. A 36-year old HIV positive man presented with a 3-week history of slowly progressive mild paresis of the left upper limb. Two days before presentation, he complained of a facial numbness on the left side and one day later a drooping corner of the mouth on the left. HIVinfection had been diagnosed 4 years previously. At presentation, CD4+ cells were 1,220/ml and HIV stage was CDC A1. The patient was on no medication and had never received antiretroviral treatment. On neurological examination, the patient had a left-sided supranuclear facial palsy in combination with slight paresis (4+/5), hypesthesia and hypalgesia of the upper left limb. There were no other abnormal neurological findings. MRI of the brain revealed multifocal lesions in the right hemisphere, suggestive of cerebral vasculitis (Fig. 1A). Lumbar puncture on the day after admission showed CSF with a mild pleocytosis with 98/3 cells (88 % lymphocytes), 40.8 mg/dl total protein and identical oligoclonal bands in CSF and serum. Serological tests for lues, borreliosis, and toxoplasmosis in serum, as well as for HSV1/2, CMV, EBV, and Enterovirus in serum and liquor were normal. Neither these viruses nor JCV could be detected in CSF by PCR. Fungal culture was negative. HIV load in plasma was 75 cop/ml, in CSF 253 cop/ml. Titers of further auto-antibodies (ANA, Anti-Phospholipid-Ab, antids-DNA-Ab) were within normal limits. Electrolytes, full blood count, lipids, inflammatory markers, and coagulation markers (PTT, TPZ-INR, APCRes, PrC, PrS, LupInh, Fbg, D-Dimer, factor VIII) were in the normal range. Toxicological screening gave no pathological findings. Neurosonological examination revealed a 50 % stenosis of the right internal carotid artery over a 4.5 cm distance immediately above the carotid bifurcation due to an irregularly configured, hypoechoic and partially fluctuating intraluminal mass. Thus, an intraluminal thrombus was suspected and was confirmed by computed tomographic angiography (CTA) (Fig. 1B, C). No hint of cardioembolic predisposition was seen in transesophageal echocardiography. No abnormality was detected by ultrasonography of the venous system of the legs and radiography of the chest. Clinical symptoms and white matter lesions could be explained by recurring thromboembolic events and thromboendarterectomy was initiated. Following surgery, treatment with clopidogrel 75 mg daily was started; 2.5 months later, the patient reported a continuous improvement of his complaints and cerebral CT confirmed that no ischemic events had occurred in the meantime. In contrast to most of the possible causes [1], the unilateral occurrence in our patient suggested an ipsilateral vascular disease as the likely cause of the lesions, whereas pleocytosis in the CSF prompted us to search for infectious or vasculitic origin. However, as demonstrated with our case, mild pleocytosis is a common finding in HIV-positive patients and should not limit the diagnostic approach [2]. Thrombosis of the internal carotid artery already has been reported in young patients with prothrombogenic factors [11], following cervical trauma and dissection of the carotid artery [9], but has sometimes remained unexplained [4]. Thrombotic microangiopathy is a well known complication in the advanced phases of HIV-infection and in association with specific conditions [8] and thrombosis has been observed in HIV-infected patients [3, 5, 10, 12]. However, against that argument, our patient had no obvious triggering cause and a normal CD4+ count. Some authors have hypothesized that proteinase-inhibitors may promote the thombogenesis [7, 13]. As the patient was on no medication, this argument would not apply in our case. Furthermore, there was no history of preceding cervical or head trauma. Further molecular genetic examination revealed heterozygosity for the PAI-1, XII and the MTHFR gene. Despite these hereditary prothrombotic components, the speLETTER TO THE EDITORS

Ictus pontino secundario a meningioma de foramen magno

Meningioma is one of the most frequent tumours affecting the central nervous system, with an estimated incidence of between 15 and 20% of all brain tumours. They are generally benign and slow growing. Their appearing as a cerebral infarction is very rare. A right-handed 75-year-old female patient with a personal history of arterial hypertension, who visited the Emergency Department because of the sudden onset of severe dysarthria, accompanied by loss of strength in the left limbs, supranuclear facial palsy on the left side and Horner syndrome on the right. A computerised tomography scan of the brain revealed a lacunar infarct in said location, with no other appreciable damage. The neurovascular study included a transcranial Doppler, which was unable to insonate the left vertebral artery or the basilar artery. Magnetic resonance imaging of the head revealed a foramen magnum meningioma, with vertebrobasilar vascular distortion. The patient progressed favourably and was submitted to radiosurgery. The case reported here is unique, since it describes a meningioma of the posterior fossa that appears in the form of the initial symptoms of a pontine stroke due to a vascular disorder.

Hemorragia cerebral en el síndrome de Sneddon: caso clínico y revisión de la bibliografía

Sneddon's syndrome is the association between livedo reticularis and stroke. Hemorrhagic strokes in Sneddon's syndrome are exceptional. A 39-year-old woman who had had a livedo reticularis for about 14 years attended the Emergency Unit complaining of sudden, severe headache and numbness and weakness in her left extremities. Physical examination revealed left supranuclear facial palsy and left crural hemiparesis and hemihypoalgesia. Cranial CT showed a right parieto-occipital lobar haemorrhage. Immunological studies, including antiphospholipid antibodies, were negative. A cerebral angiography showed anomalies in most distal branches of intracranial arteries. Biopsies of the skin and digital arteries yielded non conclusive results. The association between livedo reticularis and stroke, together with the angiographic findings led us to the diagnosis of Sneddon's syndrome. When comparing our case with similar ones, we must point out to its onset with a hemorrhagic stroke and to the lobar location of the bleeding.

Rostral lateral pontine infarction: neurological/topographical correlations.

The authors correlated neurologic features of rostral lateral pontine infarct (rLPI) with lesion location on MRI. rLPI is a motor-sensory stroke presenting as crural monoparesis or crural dominant hemiparesis and segmental superficial or deep sensory disturbances. The dorsolateral pontine base causes crural paresis without supranuclear facial palsy.

Pseudotumor inflamatorio: diagnóstico diferencial de los tumores del IV ventrículo

An inflammatory pseudotumour is a condition of unknown origin and inflammatory nature. It is rarely found in the CNS. We report a case of inflammatory pseudotumour localized to the IV ventricle and review the clinical characteristics of previously reported cases in order to outline the clinical profile of this condition. A 40 year old man was admitted to hospital complaining of a subacute condition involving difficulty in speaking and in moving his right arm and leg. On examination he had ocular deviation on initial gaze, a complete right Horner's syndrome, right supranuclear facial palsy, dyssynergy-asymmetry on the right finger-nose test and a dissociated sensitivity disorder of the left arm. On MR of the brain there was a space occupying lesion, nodular in form and fixed to the roof of the fourth ventricle. The histopathological report on the specimen removed by surgery stated it to be an inflammatory pseudotumour. On a MEDLINE search for reported cases of inflammatory pseudotumour of the CNS, 27 were found since 1967. Four cases, including ours, involved masses growing into the interior of the fourth ventricle. Inflammatory pseudotumour of the CNS is a condition affecting young adults, with a slight male predominance and some association with clinical and analytical data suggesting autoimmune dysfunction. The intraventricular site, particularly within the fourth ventricle, is relatively common (4/28) and is usually associated with clinical features of dysfunction of the posterior fossa and/or intracranial hypertension. We consider that inflammatory pseudotumour should be included in the differential diagnosis of tumours of the fourth ventricle.

Pontine supranuclear facial palsy.

Two patients presented with a unilateral supranuclear facial palsy. Additional dysarthria was attributed to the pontine origin documented by magnetic resonance imaging on the contralateral side. The pontine disorder also was indicated by an isolated delay of the blink reflex R1 component or of the masseter reflex. We attribute the facial palsy to a lesion of a supranuclear fiber bundle supplying the facial nucleus. The location of the lesions favors these fibers taking a separate course from the main pyramidal tract at the mid- to upper pontine level.

Acquired dysarthria in childhood: an analysis of dysarthric features in relation to neurologic deficits.

We analyzed the dysarthria of four children with bilateral supranuclear facial palsy and four with bilateral peripheral facial palsy. The children with peripheral lesions had only moderate dysarthria, characterized mainly by weakened vowels and consonants and by hypernasality. In contrast, children with supranuclear lesions were anarthric at first, followed by severe dysarthria with reduced stress and many pauses. Although there was a relation between severity of dysarthria and neurologic disorders, anarthria can be seen in children with bilateral supranuclear lesions and only slight neurologic disability. Damage to cortical or brainstem control mechanisms may be responsible.